Abstract

ANDRADE, Olberes Vitor B.; TROSTER, Eduardo Juan  and  CYPEL, Saul. Kinsbourne syndrome manifesting with signs of post-viral encephalitis. Rev. paul. pediatr. [online]. 2011, vol.29, n.2, pp. 300-304. ISSN 0103-0582.  http://dx.doi.org/10.1590/S0103-05822011000200024.

OBJECTIVE: To describe a case of Kinsbourne syndrome manifesting with signs of post-viral encephalitis, and to review the literature. CASE DESCRIPTION: Female child, aged two years and six months. She was referred from another hospital with a history of ataxia, irritability, and dysphasia after a prodromal episode of fever, skin and mucosa lesions. Referred with suspected post-viral encephalitis, the child was diagnosed with the opsoclonus-myoclonus-ataxia syndrome (Kinsbourne syndrome). The association of occult neuroblastoma was dismissed and therapy with corticosteroids was initiated. During hospitalization and outpatient treatment, there was a progressive regression and normalization of the clinical and neurological original condition. COMMENTS: Albeit a rare disease, the diagnosis of Kins­bourne syndrome should be recognized by pediatricians and intensivists in order to start an early specific treatment, being important to exclude occult neuroblastomas. The results of the treatment are variable.

Keywords : ataxia; mucolipidoses; ocular motility disorders; dexamethasone.